Abstract:
:Isolated congenital heart block may be associated with autoimmune disorder such as Sjögren Syndrome and systemic lupus erythematosus. In this work we demonstrate circulating autoantibodies against neonatal heart M1 muscarinic acetylcholine receptor (mAChR) in the sera of children with congenital heart block. This antibody were able to react with the second extracellular loop of the human M1 mAChR as demonstrated using a synthetic peptide in enzyme immune assay and binding assay. Affinity purified anti-peptide IgG as well as total IgG from children with congenital heart block, interfered with the specific radioligand occupancy from neonatal heart M1 mAChR, interacting irreversibly. The antipeptide antibodies also displayed an 'agonist-like' activity, i.e. decreased contractility, activated nitric oxide synthase activity and increased production of cyclic GMP. All of these effects were selectively blunted by pirenzepine and neutralized by the synthetic M1 peptide. Both binding and biological effects were obtained using neonatal rat heart instead adult heart and were independent of Ro/SS-A and La/SS-B antibodies and were also absent in the sera of normal children. A clinical relevance of these findings is demonstrated by a strong association between the existence of circulating M1 mAChR antipeptide antibodies and the presence of isolated congenital heart block, making these antibodies a proper marker of this disease.
journal_name
J Autoimmunjournal_title
Journal of autoimmunityauthors
Borda E,Sterin-Borda Ldoi
10.1006/jaut.2000.0461subject
Has Abstractpub_date
2001-03-01 00:00:00pages
143-50issue
2eissn
0896-8411issn
1095-9157pii
S0896-8411(00)90461-Xjournal_volume
16pub_type
杂志文章abstract::Antiphospholipid syndrome (APS) is characterized by thrombosis and pregnancy loss in the presence of antiphospholipid antibodies (aPL), mainly anticardiolipin, anti-beta2-glycoprotein I and lupus anticoagulant. However, similar to systemic lupus erythematosus, APS is also characterized by multiple other autoantibodies...
journal_title:Journal of autoimmunity
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journal_title:Journal of autoimmunity
pub_type: 杂志文章,评审
doi:10.1016/0896-8411(92)90016-j
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journal_title:Journal of autoimmunity
pub_type: 杂志文章
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journal_title:Journal of autoimmunity
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pub_type: 杂志文章
doi:10.1006/jaut.2001.0545
更新日期:2001-12-01 00:00:00
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journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1006/jaut.1998.0255
更新日期:1999-02-01 00:00:00
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更新日期:2019-12-01 00:00:00
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journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1006/jaut.2002.0607
更新日期:2002-11-01 00:00:00
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journal_title:Journal of autoimmunity
pub_type: 杂志文章,评审
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更新日期:2011-09-01 00:00:00
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journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1006/jaut.1996.0122
更新日期:1997-04-01 00:00:00
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journal_title:Journal of autoimmunity
pub_type: 杂志文章,评审
doi:10.1016/j.jaut.2017.08.001
更新日期:2017-11-01 00:00:00
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journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1016/s0896-8411(05)80048-4
更新日期:1992-02-01 00:00:00
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journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1016/0896-8411(89)90271-0
更新日期:1989-06-01 00:00:00
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journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1006/jaut.1996.0020
更新日期:1996-04-01 00:00:00
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journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1016/j.jaut.2006.05.001
更新日期:2006-08-01 00:00:00
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journal_title:Journal of autoimmunity
pub_type: 杂志文章,评审
doi:10.1006/jaut.1993.1015
更新日期:1993-04-01 00:00:00
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journal_title:Journal of autoimmunity
pub_type: 杂志文章
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更新日期:1991-04-01 00:00:00
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journal_title:Journal of autoimmunity
pub_type: 杂志文章
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更新日期:1999-11-01 00:00:00
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journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1006/jaut.1994.1053
更新日期:1994-10-01 00:00:00
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journal_title:Journal of autoimmunity
pub_type: 杂志文章,评审
doi:10.1006/jaut.2000.0395
更新日期:2000-09-01 00:00:00
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journal_title:Journal of autoimmunity
pub_type: 杂志文章
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更新日期:2009-08-01 00:00:00
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journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1006/jaut.2002.0596
更新日期:2002-08-01 00:00:00
abstract::Genetic polymorphism in MECP2/IRAK1 on chromosome Xq28 is a confirmed and replicated susceptibility locus for lupus. High linkage disequilibrium in this locus suggests that both MECP2 and IRAK1 are candidate genes for the disease. DNA methylation changes in lupus T cells play a central role in the pathogenesis of lupu...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1016/j.jaut.2012.12.012
更新日期:2013-03-01 00:00:00
abstract::Beta(2)-glycoprotein I (beta(2)GPI) is known as a major autoantigen for antiphospholipid antibodies. Our recent data show that binding of beta(2)GPI to oxidized low-density lipoprotein (oxLDL) or to liposomes containing anionic phospholipid(s) may facilitate the presentation of beta(2)GPI's epitope by macrophages/dend...
journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1016/j.jaut.2007.07.003
更新日期:2007-09-01 00:00:00
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journal_title:Journal of autoimmunity
pub_type: 杂志文章,评审
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更新日期:2020-07-01 00:00:00
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journal_title:Journal of autoimmunity
pub_type: 杂志文章,评审
doi:10.1006/jaut.1993.1057
更新日期:1993-12-01 00:00:00
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journal_title:Journal of autoimmunity
pub_type: 杂志文章
doi:10.1006/jaut.1997.0172
更新日期:1998-02-01 00:00:00